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how would you surgically correct an 8yo with marfans after CE for subluxated lens
leave aphakic
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what phakomatoses is not caused by a tumor supressor gene?
sturge weber syndrome (encephalotrigeminal angiomatosis) and wyburn-mason syndrome- they are both sporadic and noninherited
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what is the treatment for neonatal conjunctivitis caused by gonorrhea
"systemic ceftriaxone, vigamox if K involvement"
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what is goldenhar syndrome
"limbal dermoids, hemifacial microsomia, ear deformities, upper eyelid colobomas, vertebral anomalies"
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what is the most common location of limbal dermoids
inferotemporal limbus (think: closest to ear lobe)
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what type of cataracts associated with lowe syndrome? Alports syndrome? Downs syndrome? Galacotsemia?
"disciform cataracts, anterior subcapsular, cerulean (blue dots) cataracts, oil droplets cataract"
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what are ocular and sysetmic manifestaitons of lowe syndrome
"dense cataracts, glaucoma, corneal and conjunctival limboids; affects nervous system and kidneys (renal fanconi syndrome)"
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what causes lowe syndrome
reduction of phosphatidylinositol bisphosphate 5 phosphatase activity -> rare x-linked condition
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what is the order of surgery for primary congenital glaucoma
1. angle glaucoma surgery (goniotomy or trabeculotomy) 2. another angle glaucoma surgery 3. trabeculectomy (plus minus MMC) 4. gdi
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what is primary and secondary deviation?
"primary deviation occurs when normal eye fixing, secondary deviation when affected eye is fixing"
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"in 6th nerve palsy, is primary or secondary deviation bigger and why?"
secdonary deviation is bigger because of Herring's law (more innervation to the lateral rectus muscle results in more innervation to yoke muscle (medial rectus of other eye) resulting in larger deviation when bad eye is fixing)
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common cause of seesaw nystagmus
lesion in parasellar or midbrain area (eg. Craniopharyngioma
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common cause of downbeat nystagmus
"cervicomedullary junction (eg. Chiari type malformation), cerebellar flocculus"
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common cause of opsoclonus
neuroblastoma (usually from adrenals)
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common cause of periodic alternating nystagmus
"cervicomedullary junction (eg. Chiari type malformation), cerebellar flocculus"
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cause of upbeat nystagmus
"cerebellum, medulla, midbrain"
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cause of spasmus nutans
usually not a tumor; but similar nystagmus with parasellar/hypothalamic tumors
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what is gram negative diplococci
neisseria gonorrhea
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what is ddx for fluffy material floating around in AC after penetrating trauma?
fibrin clot which can look like liberated lens cortical material
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why dont you want to do external skin incision to decompress infected dacryocele
result in permanent lacrimal fistula
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how do you orient prisms to fix head turn in a nystagmus patient?
orient prisms to apices are at null point
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what corneal diameter do you supect glaucoma
>13mm
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what genes are resopnsbile for congenital glaucoma
"CYP1B1, GLC3A, GLC3B, GLC3C"
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what genes responsbiel for axenfeld-reiger
"PITX2, FOXC1"
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genes responsible for juvenile-onset glaucoma
GLC1A aka MYOC
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gene responsible for PXG
LOXL1
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gene responsbile for NTG
OPTN
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can you get mild retinal hemorrhage just from vaginal birth trauma
yes
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what is ophthalmia neanatorum
conjunctivitis that occurs in first month of life
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what is chemical conjuncitivitis
occurs from silver nitrate drops in first 24 hrs of life
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when does gonorrhea conjuncitivitis occur
"usually 3-4 days, but can be 3 weeks after birth"
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when does chlamydia conjuncitivis present
7 day sof life; earlier if rupture of maternal amniotic membranes
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when does hsv conjuncitivitis occur in a kid
2 weeks; rare
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what is white eyed blowout fx
elevation and depression deficiencies without significant soft tissue swelling due to entrapment -> needs immediate surgery
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what surgery would you do for type 1 duanes and face turn and why
medial rectus recession of involved eye; lateral rectus resection can worsen globe retraction
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is brown a or v pattern? Inferior oblique paralysis a or v pattern?
"V pattern (think victor brown), A pattern"
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what is a potential cause of acute onset browns in adult
sinusitis
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what is de morsier syndrome
"optic nerve hypoplasia, absent septum pellucidium, hypopituitarism (usually GH deficiency, but can also be ACTH, ADH, TSH, LH, FSH)"
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how does teller preferential looking card work?
card has stripes and gray background on left and right side; series of cards where stripes become finer and finer until kid cant distinguish between the two sides
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how do you manage the A or V pattern in exotropia with R+R (how do you displace muscles)
"displace medial rectus resectino towards apices of A or V (up in A and down in V), and lateral rectus towards open area"
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what cn involved in macrus gunn jaw winking
III and V
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is heridtary or sporadic form of aniridia associated with wilm tumor
sporadic form; hereditary form not associated
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what are associations of anirdiia
"foveal hypoplasia, frequent nystagmus most common; others: glaucoma, ON hypoplasia, cataracts, diffuse K scarring from limbal stem cell deficiency"
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what gene mutation inolved in aniridia
pax6
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what are signs of parinaud's syndrome or dorsal midbrain syndrome
"paralysis of upgaze, defective convergence, pupillary light near dissociation, convergence-retraction nystagmus, eyelid retraction (""collier's sign"")"
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what causes parinaud's syndrome
"pineoloma and congenital aqueductal stenosis; MS in young adult women, midbrain stroke in elderly"
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"what are average convergence fusional amplitudes, divergence fusional amplitudes, and verticfal fusional amplitudes"
14 PD; 6 PD; 2.5 PD
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what is alexander's law
nystagmus is more pronounced when gaze is directed at side of fast beating component
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What is donder's law
every tertiary eye rotation is associated with a specific torsional rotation
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what is sherrington's law
contractino of a given muscle is associated with a decrease in contraction of it's antagnoist
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what violates sherrington's law
duane's (co-contractino of medial and lateral rectus can occur)
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what is herring'tons law
yoke muscles receive equal innervational input
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what violates herringtons' law
dvd
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what are differences between akc and vkc
vernal affects superior conj while akc affects both sup and inferior conj; both affects more males; VKC has seasonal preference while akc is yearround
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what is monofixation syndrome
peripheral fusion without bimacular fusion (due to macular scotoma)
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how do you diagnose monofixation
"w4d at 10 feet, pt will not have fusion, but closer than that, patient has fusion (since light will project onto peripheral retina); stereo will be 200 or worse"
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what are classic signs of homocystinuria
"ectopia lentis, mental retardation and history of thrombotic events; other things include marfanoid habitus, pectus excavatum, seizures, myopia, glaucoma, optic atrophy"
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how do you treat homocystinuria
"high doses of vit B6, folate, pyridoxine and low methionine diet"
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why is inferior oblique advancement dangerous
can perforate macula since you are moving it posteriorly
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why would you want to give full hyperopic correction to ambylopic eye
amyblopic eye can't accommodate effectively
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what is leber's congenital amarousis
AR disorder of rods and cones; thought to be more severe form of RP
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which way is the head turn in latent nystagmus
"head turn away from occluded eye because the patient will look toward the occluded eye to find the null point (since fast phase is toward the eye that is NOT occluded, which results in a right jerk nystagmus when left eye is occluded)"
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what is the characteristic waveform of latent nystagmus vs convenital motor nystagmus
exponential decrease in slow wave velocity in latent; exponential increase in slow wave velocity in CMN
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what is the classic neurological presentation of sturge weber syndrome
stroke like episode occuring in young child after minor head trauma
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what are classic findings of sturge weber syndrome
1. not genetically transmitted 2. port wine stain 3. secondary glaucoma from increased episcleral venous pressure on side of port wine stain
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"you see a diffuse choroidal hemangioma in a kid, what phakomatoses?"
sturge weber
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what are classic findings of sulfite oxidase deficiency
"recurrent seizures, hypertonia, deep set eyes"
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what are all causes of ectopia lentis
"marfan, homocystinuria, sulfite oxidase deficiency, syphillis, ehlos danlos, trauma, aniridia, iris coloboma, congenital glaucoma, hereditary ecotpia lentis, hyperlisenemia"
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what is dominant optic atrophy
"AD condition with bilateral temporal pallor, slow loss of vision and absent nystagmus -> visual potential is usually ok 20/200 is worse; associated with OPA1 gene"
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what can inferior rectus recession cause to eyelid? resection?
lower lid retraction and thus widening of palpebral fissure; opposite for resection; this is due to extensive connections between inferior retus muscle and lower eyelid
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what muscles have longest tendons? shortest overall muscle? shortest active muscle portion?
superior oblique and levator; inferior oblique; superior oblique
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what is the most common cause of amyblopia in congenital ptosis
anisometropic (most ptosis dont involve pupillary axis and kids can do a chip up position)
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what is the kestenbaum-anderson procedure
shift the null point closer to primary position by moving the eyes away from the null point and toward the head turn
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what antiemetic is CI for kids < 2yo and why?
promethazine (phenergan); can cause respiratory depression and even death
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what is triad for congenital glaucoma
"epiphora, blepharospasm, photophobia"
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what is the test to monitor response to treatment for congenital glaucoma
"A-scan ultrasound because if IOP is poorly controlled, can result in larger eye"
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